University of Stellenbosch. Faculty of Health Sciences. Dept. of Psychiatry.

dc.date.accessioned

2008-08-04T10:05:41Z

en_ZA

dc.date.accessioned

2010-06-01T08:19:35Z

dc.date.available

2008-08-04T10:05:41Z

en_ZA

dc.date.available

2010-06-01T08:19:35Z

dc.date.issued

2006-12

en_ZA

dc.identifier.uri

http://hdl.handle.net/10019.1/1362

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Thesis (PhD (Psychiatry))--University of Stellenbosch, 2006.

dc.description.abstract

Lipoid Proteinosis (LiP) is a rare hereditary disease, which often results in bilateral,
symmetrical and circumscribed calcifications in the mesial temporal region (especially
the amygdala). While several case studies have been published on individuals with this
illness, there have been few systematic investigations of the neuropsychiatry and
neuropsychology of a series of patients. Thirty-seven LiP patients were extensively
assessed with standardized neuropsychiatric and neuropsychological measures. Of
these, 27 patients from the Northern Cape in South Africa were matched (for age,
gender, education, language, geographical area) with 53 controls. There was a high
incidence of neuropsychiatric disorders in LiP (more than half of the subjects reported a
history of depression or anxiety and 12% had a diagnosis of schizophrenia). Despite a
wide variance, LiP subjects performed poorly on facial recognition for emotions and on
most neuropsychological measures including intelligence, recall and executive
functioning. These findings are consistent with involvement of the mesial temporal areas
in mood, anxiety, and psychotic symptoms, and in the cognitive-affective processes.
Future work aimed at delineating the associations between the clinical and
neuropsychological findings reported here, for example, with brain-imaging techniques,
is needed.